Extramammary Paget Disease of the Scrotum With Features of Bowen Disease

Abstract

The scrotum is an uncommon site for the presentation of extramammary Paget disease (EMPD). We describe a case of EMPD that was discovered in a patient who had been previously diagnosed and treated for squamous cell carcinoma in situ of the scrotum 3 years earlier. Pathologic examination of the current scrotectomy specimen revealed an erythematous patch with areas of pale induration. Microscopic examination revealed areas with the characteristic histology of Paget disease adjacent to areas characteristic of Bowen disease. Immunohistochemical findings demonstrated a strong expression of carcinoembryonic antigen, cytokeratin 7, and low-molecular-weight cytokeratins (CAM 5.2) in both of these areas, giving support to the overall diagnosis of EMPD. High-molecular-weight cytokeratins (34βE12) were uncharacteristically expressed in the cytoplasm of the Paget cells with equal or greater strength than in the surrounding keratinocytes, suggesting some degree of squamous differentiation. Very few publications have reported the coexistence of EMPD with squamous cell carcinoma in situ, occurring mostly in the vulva. To our knowledge, our case is the first report of scrotal EMPD with features of Bowen disease. Our findings support the theory that primary EMPD arises multifocally from multipotential epidermal cells.

Anogenital syringocystadenocarcinoma papilliferum in situ or anaplastic extramammary Paget disease? A unifying concept and review of the literature

“…Histopathologically, the lesions exhibited “cleft like” acantholysis with full‐thickness atypia, mimicking acantholytic squamous cell carcinoma in situ 16 . Two additional cases of PD with similar features were reported in 2009, 17 and since that time similar findings have been reported in cases of EMPD under various descriptions, including acantholytic anaplastic EMPD, EMPD mimicking acantholytic squamous cell carcinoma in situ, and syringocystadenocarcinoma papilliferum in situ (SCACPIS)‐like change 16‐29 . In addition to the findings of these atypical cases of EMPD with full‐thickness bowenoid atypia and acantholysis, our case also notably overlaps with prior descriptions of SCACPIS, including prominent glandular formation and involvement of adnexal structures with papillations and a plasma‐cell‐rich infiltrate.…”

“…To avoid diagnostic confusion and in keeping with historical initial descriptions of the findings in our case, we suggest applying the terminology of “anaplastic EMPD” or “EMPD with anaplastic features” to cases of EMPD with findings previously described under SCACPIS‐like change in EMPD, acantholytic anaplastic EMPD, EMPD mimicking acantholytic SCCIS, and anogenital SCACPIS, including: full‐thickness bowenoid atypia, acantholysis, glandular formation, adnexal involvement, and anogenital site 16‐29 . Adoption and application of consistent terminology would help to solidify knowledge and awareness of this histopathologic variant.…”

“…Of additional interest, multiple cases of SCACPIS have been described in the anogenital region 17‐21 . Despite the variations in interpretations of findings, the aforementioned cases of EMPD and anogenital SCACPIS share similar histopathologic and clinical features—likely representing findings on a spectrum of the same entity—and are summarized in Table 1 19‐29 …”

Treatment Outcomes of Adjunct and Stand-alone Photodynamic Therapy in Patients With Extramammary Paget Disease: A Literature Review

“…4 EMPD does not have a uniform histogenesis, with 2 theories that underlie its current pathogenesis. 5 The first theory proposes that the intraepidermal origin of Paget cells have the potential to be associated with an apocrine gland carcinoma; the second theory proposes that it arises from the metastasis of Paget cells into the epidermis from the underlying apocrine glands. 6 The exact incidence of EMPD is not known but is estimated to account for 1% to 6% of all Paget disease cases.…”